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From the Departments of Neurology (Drs. Bosboom, Van den Berg, De Boer, and Van Son, H. Veldman, and Dr. Wokke) and Clinical Neurophysiology (Dr. Franssen) of the Rudolf Magnus Institute for Neurosciences; and Department of Immunology (Dr. Logtenberg), University Hospital Utrecht, the Netherlands.
Address correspondence and reprint requests to Dr. L.H. Van den Berg, Department of Neurology, University Hospital Utrecht, P.O. Box 85500, 3508 GA Utrecht, the Netherlands.
BACKGROUND: T-cell infiltrates in sural nerve biopsy specimens of patients with inflammatory neuropathies have been reported, suggesting a role for T cells in the pathogenesis, but the specificity of the presence and localization of sural nerve T cells in chronic inflammatory demyelinating polyneuropathy (CIDP) is unknown.
OBJECTIVE: To study the diagnostic value of the number and distribution of sural nerve T cells in CIDP.
METHODS: We performed a quantitative immunohistochemical examination of T cells in sural nerve biopsy specimens taken from 23 patients with a CIDP and compared them with sural nerves of 15 patients with a chronic idiopathic axonal polyneuropathy (CIAP), 5 patients with a vasculitic neuropathy, and 10 normal controls.
RESULTS: T cells were found in sural nerves of all CIDP patients as well as in all disease and normal controls. Only six CIDP patients had increased numbers and densities of T cells compared with CIAP patients and controls. Based on the distribution of endoneurial or epineurial T cells, it was not possible to differentiate CIDP patients from CIAP patients or normal controls. In patients and controls perivascular epineurial T cells predominated. Increased numbers and densities of sural nerve T cells in patients with CIDP were associated with female sex, a more severe disease course, worse outcome, highly elevated CSF protein level, and a larger sural nerve area, but not with loss of myelinated nerve fibers in the sural nerve biopsy sample or demyelinating features on electrophysiologic examination.
CONCLUSIONS: In the majority of CIDP patients, the number and distribution of T cells in sural nerve biopsy samples were similar to patients with noninflammatory neuropathies and normal controls. Only large numbers of sural nerve T cells are specific for inflammatory neuropathies and therefore of diagnostic value for CIDP.
Key words: Chronic inflammatory demyelinating polyneuropathySural nerveT cell.
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